20 research outputs found
The First VERITAS Telescope
The first atmospheric Cherenkov telescope of VERITAS (the Very Energetic
Radiation Imaging Telescope Array System) has been in operation since February
2005. We present here a technical description of the instrument and a summary
of its performance. The calibration methods are described, along with the
results of Monte Carlo simulations of the telescope and comparisons between
real and simulated data. The analysis of TeV -ray observations of the
Crab Nebula, including the reconstructed energy spectrum, is shown to give
results consistent with earlier measurements. The telescope is operating as
expected and has met or exceeded all design specifications.Comment: Accepted by Astroparticle Physic
A newborn with overlapping features of AEC and EEC syndromes
Item does not contain fulltextEctrodactyly, ectodermal dysplasia, clefting (EEC) syndrome is the prototype of several p63 conditions, which include ankyloblepharon, ectodermal dysplasia, clefting (AEC) syndrome, limb-mammary syndrome (LMS), Rapp-Hodgkin syndrome (RHS), ADULT syndrome, and others. All these disorders include combinations of ectodermal dysplasia, orofacial clefting and limb malformations in variable severity. A newborn patient is presented with diffuse erythematous and desquamating skin lesions and anal atresia. She also had sparse and lightly colored thin hair, deeply set eyes, hypoplastic alae nasi, and a short philtrum. Cleft lip/palate and ankyloblepharon were not present. Complete cutaneous syndactyly was present on both hands in between the third and fourth fingers. Mild ectrodactyly was evident on all four extremities in between first and second digits. There was post-axial polydactyly on both feet. Anal atresia was present and defecation occurred through a rectovaginal fistula. The patient represented an interesting overlapping clinical condition between AEC and EEC syndromes. Diffuse skin lesions with excoriation and desquamation suggest AEC syndrome, despite the absence of ankyloblepharon, however; ectrodactyly and polydactyly strongly suggest the EEC syndrome. C308Y mutation in exon 8 of TP63 gene was detected, which was previously described to lead only to EEC syndrome and not to any of the other allelic conditions. These data emphasize the large degree of clinical variability that may be seen for specific TP63 mutations. (c) 2011 Wiley Periodicals, Inc